Progress in
Cardiovascular Diseases MAY/JUNE 1975
VOL. XVII, NO. 6
The Patient With Congenital Heart Disease After Surgical Repair: An Overview Amnon Rosenthal
HE ERA of surgical treatment of congenital cardiovascular disease was ushered in when Dr. Robert Gross successfully ligated a patent ductus arteriosus in 1938. During the subsequent two decades, corrective surgery was performed on patients with relatively simple cardiovascular malformations; such as coarctation of the aorta, atrial septal defect or pulmonary stenosis, and palliative surgery in patients with more complex lesions such as tetralogy of Fallot or complete transposition of the great arteries. In the last decade, the surgeons ingenuity and improved techniques have resulted in successful repair of the most complex malformations. Transposition of the great arteries, tricuspid atresia, and complete atrioventricular canal, for example, have become amenable to surgical repair. Recently, the most profound and far reaching changes have been the introduction of one stage surgical correction in infants instead of palliation followed by correction in childhood. Malformations such as ventricular septal defect, tetralogy of Fallot, and transposition of the great arteries can now be corrected in infancy utilizing deep hypothermic circulatory arrest. Corrective surgery in the neonate and infant has significantly altered the pattern of care and quality of life. Survival and relief of symptoms are the immediate goal of surgery. The long term objective of surgical and medical treatment for infants and
T
From the Departments of Cardiology and Pediatrics, The Children's Hospital Medical Center, Harvard Medical School, Boston, Mass. Reprint requests should be addressed to Amnon Rosenthal, M.D., Senior Associate in Cardiology, The Children's Hospital Medical Center, 300 Longwood Ave., Boston, Mass. 02115. 9 1975 by Grune & Stratton, Inc.
children with cardiovascular malformations remains to provide optimal physical, intellectual, and emotional growth that will enable them to be healthy adults. Many of the children who have undergone surgical correction or palliation have reached adulthood. They present new and challenging clinical and laboratory findings with which the internist or the adult cardiologist may not be familiar. For example, pulmonary regurgitation is commonly present in postoperative patients with tetralogy of Fallot and right bundle branch block is nearly a uniform electrocardiographic pattern in patients who have had a right ventriculotomy for correction of tetralogy of Fallot or ventricular septal defect. Awareness of the cardiac and extracardiac complications, sequelae, and residua that follow surgery will permit the physician to assess the significance of the clinical findings and laboratory studies observed. Evaluation of the cardiovascular status in the postoperative patient may require exercise testing, electrophysiologic, or myocardial function studies by cardiac catheterization in addition to the customary examination, chest x-ray, and electrocardiogram. Management may differ considerably from the nonoperated patient with congenital heart disease. The majority of postoperative patients can lead a normal family life. However, with few exceptions, they require closer medical supervision than other young adults. Susceptibility to infective endocarditis persists in most postoperative patients. They also should be given recommendations regarding recreational and occupational activities and may require genetic counseling. Women who have reached childbearing age may be expected to have an increased frequency of offsprings with
Progress in Cardiovascular Diseases, Vol. XVII, No. 6 (May/June), 1975
401
402
congenital cardiac malformations. How well these patients would tolerate hypertensive or coronary artery disease remains unknown. I would suspect that these may have a more adverse effect on the postoperative patient with congenital heart disease because of the frequent presence of conduction or rhythm abnormalities, poorer myocardial function, or injury associated with previous prolonged volume or pressure overload, chronic hypoxemia, or residual pulmonary vascular obstructive changes. The purpose of this symposium is to provide the internist and cardiologist with up-to-date informa-
AMNON ROSENTHAL
tion on the clinical and laboratory findings, course, management, and prognosis of the postoperative patient with congenital heart disease. The individual manuscripts review problems common to many postoperative patients, the diagnostic tools utilized and, when appropriate, include an in depth discussion of specific problems associated with the common cardiac lesions. In many instances, particularly with regard to the long-term prognosis of the very complex cardiac lesions, only scant information is available and further recommendations must await more clinical research.
Cardiovascular Diseases MAY/JUNE 1975
VOL. XVII, NO. 6
The Patient With Congenital Heart Disease After Surgical Repair: An Overview Amnon Rosenthal
HE ERA of surgical treatment of congenital cardiovascular disease was ushered in when Dr. Robert Gross successfully ligated a patent ductus arteriosus in 1938. During the subsequent two decades, corrective surgery was performed on patients with relatively simple cardiovascular malformations; such as coarctation of the aorta, atrial septal defect or pulmonary stenosis, and palliative surgery in patients with more complex lesions such as tetralogy of Fallot or complete transposition of the great arteries. In the last decade, the surgeons ingenuity and improved techniques have resulted in successful repair of the most complex malformations. Transposition of the great arteries, tricuspid atresia, and complete atrioventricular canal, for example, have become amenable to surgical repair. Recently, the most profound and far reaching changes have been the introduction of one stage surgical correction in infants instead of palliation followed by correction in childhood. Malformations such as ventricular septal defect, tetralogy of Fallot, and transposition of the great arteries can now be corrected in infancy utilizing deep hypothermic circulatory arrest. Corrective surgery in the neonate and infant has significantly altered the pattern of care and quality of life. Survival and relief of symptoms are the immediate goal of surgery. The long term objective of surgical and medical treatment for infants and
T
From the Departments of Cardiology and Pediatrics, The Children's Hospital Medical Center, Harvard Medical School, Boston, Mass. Reprint requests should be addressed to Amnon Rosenthal, M.D., Senior Associate in Cardiology, The Children's Hospital Medical Center, 300 Longwood Ave., Boston, Mass. 02115. 9 1975 by Grune & Stratton, Inc.
children with cardiovascular malformations remains to provide optimal physical, intellectual, and emotional growth that will enable them to be healthy adults. Many of the children who have undergone surgical correction or palliation have reached adulthood. They present new and challenging clinical and laboratory findings with which the internist or the adult cardiologist may not be familiar. For example, pulmonary regurgitation is commonly present in postoperative patients with tetralogy of Fallot and right bundle branch block is nearly a uniform electrocardiographic pattern in patients who have had a right ventriculotomy for correction of tetralogy of Fallot or ventricular septal defect. Awareness of the cardiac and extracardiac complications, sequelae, and residua that follow surgery will permit the physician to assess the significance of the clinical findings and laboratory studies observed. Evaluation of the cardiovascular status in the postoperative patient may require exercise testing, electrophysiologic, or myocardial function studies by cardiac catheterization in addition to the customary examination, chest x-ray, and electrocardiogram. Management may differ considerably from the nonoperated patient with congenital heart disease. The majority of postoperative patients can lead a normal family life. However, with few exceptions, they require closer medical supervision than other young adults. Susceptibility to infective endocarditis persists in most postoperative patients. They also should be given recommendations regarding recreational and occupational activities and may require genetic counseling. Women who have reached childbearing age may be expected to have an increased frequency of offsprings with
Progress in Cardiovascular Diseases, Vol. XVII, No. 6 (May/June), 1975
401
402
congenital cardiac malformations. How well these patients would tolerate hypertensive or coronary artery disease remains unknown. I would suspect that these may have a more adverse effect on the postoperative patient with congenital heart disease because of the frequent presence of conduction or rhythm abnormalities, poorer myocardial function, or injury associated with previous prolonged volume or pressure overload, chronic hypoxemia, or residual pulmonary vascular obstructive changes. The purpose of this symposium is to provide the internist and cardiologist with up-to-date informa-
AMNON ROSENTHAL
tion on the clinical and laboratory findings, course, management, and prognosis of the postoperative patient with congenital heart disease. The individual manuscripts review problems common to many postoperative patients, the diagnostic tools utilized and, when appropriate, include an in depth discussion of specific problems associated with the common cardiac lesions. In many instances, particularly with regard to the long-term prognosis of the very complex cardiac lesions, only scant information is available and further recommendations must await more clinical research.
